Obstructed hemi-vagina and ipsilateral renal anomaly syndrome in Vietnam: an overview from clinical diagnosis approach to management: a case report

Abstract

Background

Obstructed hemi-vagina and ipsilateral renal anomaly syndrome is a rare congenital condition, which is described in a combination of uterus didelphys and obstructed hemi-vagina with ipsilateral renal agenesis. Until now, the diagnosis of this anomaly is challenging as there are diverse symptoms depending on pre-puberty and post-puberty age onset. Early diagnosis of this syndrome allows for improvement of quality of life and prevents fertility-related complications.

Case report

We present the case of a 13-year-old Vietnamese girl who was admitted to the emergency department with colicky lower abdominal pain and a tender cystic mass in the left vaginal wall. Her menarche started 6 months ago, and she had an experience of progressive menstruation-related pain. The ultrasound and magnetic resonance imaging identified presence of uterus didelphys, an absence of left kidney associated with a menstrual-blood-retained mass from the uterus cavity to the left side of vagina (hematocolpos). The patient underwent a vaginal excision to drain blood-containing mass and was discharged from hospital 5 days after the operation. The patient and her parents have been provided with a detailed follow-up plan to prevent the complications of renal failure and infertility.

Conclusion

The possibility of obstructed hemi-vagina and ipsilateral renal anomaly syndrome should be considered in both pre-puberty and post-puberty girls who present with acute lower abdominal pain. Ultrasound plays a crucial role in making an early diagnosis of this condition, while magnetic resonance imaging allows for classification and prediction of fertility capacity. Vaginal excision is a minimally invasive procedure to relieve the pain and prevent the adverse effects.

Background

Obstructed hemi-vagina and ipsilateral renal anomaly (OHVIRA) syndrome, also known as Herlyn–Werner–Wunderlich (HWW), is a very rare congenital anomaly of the genitourinary system with the prevalence varying from 0.1–3.8% [1, 2]. As the name suggests, this syndrome is related to the abnormality of Mullerian and Wolffian duct development [3, 4], and characterized by a triad of uterus didelphys with blind hemi-vagina and ipsilateral agenesis [1]. Clinical findings have been reported in different clinical contexts throughout either urinary or genital disorders depending on the pre-puberty and post-puberty age of onset [5–8]. Nevertheless, early diagnosis plays an important role in establishing immediate interventions to improve quality of life and prevent complications related to urinary tract infections and future fertility.

In literature, the OHVIRA syndrome has been documented in many ethnic groups in Europe, the Middle East, Asia, and Latin America [5, 6, 9–12], but none has been reported in Vietnam. This is the first case report of OHVIRA syndrome in a Vietnamese teenager, who presented to the emergency room because of acute colicky lower abdominal pain. This report aimed to give an overview of the diagnosis approach on the basis of clinical and imaging findings, then establish an appropriate management and follow-up to preserve future fertility.

Case presentation

A 13-year-old Vietnamese girl was admitted in the emergency department of University Medical Center—Branch 2, Ho Chi Minh city, Vietnam because of colicky lower abdomen pain on day 5 of menstruation. Her menarche started 6 months ago, when she experienced progressive abdominal pain throughout the 7 days of menstruation, which required her to take 4–5 ibuprofen tablets per day to deal with the pain. She had not been sexually active yet. There was no associated fever or vomiting noticed. The patient did not have a remarkable medical history in her family.

A general examination found normal ranges. Her body max index (BMI) was 18, and secondary genital characteristics including development of breasts and hair distribution in armpits and pubic areas showed consistent with her age. An abdominal palpation revealed a tender mass in the lower abdomen that was very painful. When performing a digital rectal examination, a stressed, bulky, and non-limited liquid mass was detected on the left side in the posterior wall of vagina.

Abdominal ultrasound determined unique right kidney (Fig. 1), in addition to uterus didephys with size of 25 mm (right) and 21 mm (left), measured with anteroposterior diameters. Endometrium had the same thickness of 2 mm in both uteri. Both ovaries were found to be normal size. Heterogeneous mass measuring 59.5 × 97.4 mm was also identified from the left uterus cavity to the left vaginal wall, suggesting obstructed hemi-vagina (Fig. 2 and 3).

Fig. 1.

Right kidney (A Longitudinal section, B traversal section) and absence of left kidney

Fig. 2.

Pelvic mass found from the left uterus cavity to the left vaginal wall, suggesting obstructed hemi-vagina

Fig. 3.

Blood retained mass (red star) extended from left uterus cavity to the left side of the vagina in sagittal plan in magnetic resonance imaging T1 (A) and magnetic resonance imaging T2 (B)

Other laboratory tests including renal function (creatinine and estimated glomerular filtration rate (eGFR)) showed within the range of normal values.

In addition to the ultrasound, magnetic resonance imaging (MRI) was also indicated to confirm the presence of left-sided obstructed hemi-vagina causing a large fluid-filled mass from the left uterus cavity to the left vaginal wall (Fig. 4), combining with uterine didelphys (Fig. 5) and only one kidney on the right side (Fig. 6). The finding results matched well with the ultrasound diagnosis.

Fig. 4.

Uterus didelphys (U1 and U2) and blood-retained mass (red star) in traversal plan in magnetic resonance imaging

Fig. 5.

Unique right kidney in magnetic resonance imaging in T1 (A) and T2 (B)

Fig. 6.

Vaginal examination showing the septum (arrow) (A) and vaginal examination using two valves to inspect the vaginal septum (B)

After diagnosis of OHVIRA syndrome had been confirmed, the minimally invasive procedure was explained to the patient and her parents, which included excising the vaginal septum to relieve the obstruction and draining the hematocolpos, which was the main reason of lower abdominal pain.

A vaginal examination had been performed under anesthesia in the operation room. There was a longitudinal septum separated completely from the vagina. However, this septum was terminated in the middle of the left vaginal wall near to the vaginal entrance on the left side of the body (Fig. 6) and obstructed the observation of cervix on the left side, whereas it was easy go through the vagina in the right side of the body to demonstrate the right cervix connected to the right uterine.

Treatment procedure was started by excising vaginal septum and drainage of the hematocolpos in the left vaginal cavity through fine-needle aspiration. After draining a total of 150 mL of dark brown liquid and washing with normal saline, the vaginal septum was resected from the left vaginal wall and opened a second entrance to connect with the left cervix. The edge of the excised wound was sutured by chronic vicryl 5.0

The patient was discharged from the hospital on day 5 after vaginal excision. At 1-month follow-up examination, no recurrence of pain during menstrual period was reported from the patient. The patient and her parents were provided with a regular 1-year follow-up plan with a family doctor and gynecologist to check renal function through blood test and gynecological ultrasound to screen the ovarian mass (endometrioma) or adnexal adhesion (hydrosalpinx).

Discussion and conclusion

OHVIRA syndrome, also known as Herlyn–Werner–Wunderlich syndrome, has been described as a triple genitourinary anomaly consisting of uterine didelphys, unilateral obstructed hemi-vagina, and ipsilateral renal agenesis [1, 2]. This is a rare congenital condition related to an abnormal development of Mullerian and Wolffian ducts, with incidence reported from 0.1% to 3.8% in female individuals [1]. The etiology of this malformation remains unknown [3, 4].

In terms of clinical practice, the diagnosis of this syndrome is challenging as there are various clinical symptoms reported depending on the onset age of pre-puberty or post-puberty in girls. Focusing on the age of onset, Zhu et al. has identified the common age of detection in OHVIRA syndrome, which is found to be between 13 and 25 years of age [5, 7]. Nevertheless, the onset age of this syndrome can appear from the very early days of birth (1-day-old newborn) [6] to late reproductive age (in 34-year-old woman) [8]. Sijmons et al. has described a 1-day-old newborn girl who suffered from anuria and acute pyelonephritis, resulting from cystic kidney associated with ipsilateral dilated ureter [6]. The presence of hydrocolpos caused a cystic pelvic mass detected subsequently by physical and abdominal ultrasound [6]. The earlier age of onset occurs, the more acute and serious clinical symptoms show up, and the most common findings are related to urinary disorder [6], whereas OHVIRA syndrome has been demonstrated by dormant symptoms in the reproductive age women, and it becomes visible distinctly through its complications, such as infertility and dysmenorrhea [8].

To better recognize this syndrome, Zhu et al. divided the syndrome into two principal clinical types: completely obstructed hemi-vagina (with/without communicating uteri), called classification 1, and incompletely obstructed hemi-vagina (with/without communicating uteri), called classification 2 [5] (Fig. 7, 8). This classification is very pragmatic in clinical practice since it allows frontline healthcare professionals to identify OHVIRA syndrome earlier on the basis of age of finding onset. Generally, female individuals who suffer from the classification 1.1 (completely obstructed hemi-vagina) have an earlier age of onset (before menarche) and present with kidney-related dominant symptoms [5]. Conversely, women in the classification 2 (incompletely obstructed hemi-vagina) will have a later onset after menarche and appear with genital-based findings [5].

Fig. 7.

Classification 1.1, with blind hemi-vagina and 1.2 (A), with cervicovaginal atresia, without communicating uteri (B) [5]

Fig. 8.

Classification 2.1, with partial reabsorption of the vaginal septum and 2.2 (A), incompletely obstructed hemi-vagina with communicating uteri (B) [5]

Eventually, noninvasive diagnostic tests, such as abdominal ultrasound and MRI, are crucial tools to diagnose and classify this syndrome. Ultrasound allows for easy detection of both uterine (uterus didelphys) and urinary (renal agenesis) malformations, identifying exactly the character of fluid-filled mass [13]. In addition, MRI evaluates more detailed soft tissue structures, especially uterine and vagina morphology as well as the fluid content nature, which are necessary to classify OHVIRA syndrome and determine its classification [5, 14]. Therefore, ultrasound should be used as the firsthand device to diagnose OHVIRA syndrome, followed by MRI to determine the syndrome’s classification [13, 14].

Our case is the first description of OHVIRA syndrome in Vietnam. The patient was a teenager, whose age was found between the transition of pre-puberty and post-puberty period (13-year-old girl). The girl was admitted to the emergency room following the context of an acute lower abdominal pain with a presence of tender pelvic mass, which suggested either a twisted ovary or ruptured ovarian follicular. Clinical examination revealed a large tender cystic mass in the left cervical–vaginal area, and an abdominal ultrasound identified hematocolpos with uterus didelphys that helped to rule out ovary-induced emergency issues. OHVIRA syndrome was confirmed after MRI, in which a completely obstructed hemi-vagina on the left side associated with ipsilateral renal agenesis was identified. The present case was compliant with typical OHVIRA syndrome in classification 1.1 (completely obstructed hemi-vagina associated with blind hemi-vagina) [5]. It is reasonable to understand the disease progression that brings the patient to the emergency department. In this patient, 6 months after the menarche is the cutoff timepoint at which hematocolpos accumulates increasingly and becomes visible.

Regarding the affected side, Vercellini et al. stated that the prevalence of right-sided anomaly in OHVIRA syndrome is twofold higher than left-sided [12]. In our case, the left side was affected, similar to the recent case reported by Elgohary et al. in the United Arab Emirates in 2023 [9] and that of Horst et al. in Brazil in 2021 [10]. Although there is a little difference in onset age, a post-pubertal 11-year-old girl of Middle East ethnicity [9] and a 14-year-old of Latin American ethnicity [10], these adolescent girls were admitted to the emergency clinical picture due to colicky lower abdominal pain caused by OHVIRA syndrome on the left side in the 11-year-old girl and on the right side in the 14-year-old girl, in addition to ipsilateral renal abnormality [9, 10]. Both reported cases described preliminary acute lower abdominal pain associated with a bulge of hematocolpos [7, 9, 10], with a short period of menstrual blood accumulation, consistent with classification 1.1 (blind hemi-vagina).

In literature, OHVIRA syndrome’s etiology is still unknown, and the evidence of direct genetic causation has not yet been established [3–5, 7]. In terms of clinical practice, an early diagnosis to set up an appropriate treatment plays a more important role since it would help to relieve the hematocolpos-induced pain and prevent the progression of complications [5, 9, 13, 14]. Obviously, a prolonged menstrual outflow obstruction may lead to retrograde blood flow causing endometriosis, endometrioma, and peritonitis, which could possibly adversely affect women’s future fertility [7, 10, 11, 14]. In some studies, urinary tract infections were also reported because the hematocolpos constantly compresses the bladder [6, 10, 15], especially in pre-puberty patients [6, 15]. That is why Del Vescovo et al. has recommended routine investigation of the urinary tract whenever OHVIRA syndrome has been established, since there is a high risk of renal failure and urinary tract infection [1, 13]

In the present case, the patient was carefully screened for urinary complications at the beginning through blood test (GFR and creatinine), urine test (ten parameters), and ultrasound. The results were normal range. However, the patient and her parents were informed about the potential risks associated with impaired kidney function and retrograde infections. They were advised to have a regular follow-up plan managed by the family doctor to ensure proper function of the right kidney. Additionally, an annual consultation with a gynecologist was recommended to monitor for possible endometriomas, endometriosis, and potential fertility-related issues, due to the patient’s experience with menstrual blood retention.

OHVIRA syndrome treatment is always focused on two principal purposes: alleviation of pain and preservation of future fertility through relieving obstruction [5, 7, 12]. Therefore, transvaginal resection of the obstructed septum in blind hemi-vagina is the most appropriate treatment of OHVIRA syndrome [6, 7, 16]. In patients who wanted to keep hymen integrity, a two-step approach is required, in which the excision of the vaginal septum under general anesthesia is performed as the first step, followed by hymen repair as the second step [7, 10, 17]. A laparoscopy is not mandatory but is suggested to evaluate whether there is menstrual blood regurgitation or pelvic adhesion and endometriosis, especially when MRI findings are inclusive [11, 13]. A minimally invasive approach was chosen to excise the vaginal septum and drain the hematocolpos in this case because there was no evidence of endometrioma and endometriosis in radiological images. When the diagnosis and treatment of OHVIRA syndrome were promptly conducted, the prognosis was relatively good. Before discharge from hospital, the girl and her parents received a detailed follow-up plan at 1-month postoperation and 1-year interval visits to screen for possible complications related to renal and fertility function.

OHVIRA syndrome is a very rare congenital genital–urinary malformation, but early diagnosis of OHVIRA helps to prevent the progression of complications. The first case report of this syndrome in Vietnam has helped Vietnamese frontline clinical physicians to be able to recognize this condition and provide an appropriate treatment for preserving the patients’ fertility.

Abbreviations

BMI

Body mass index

MRI

Magnetic resonance imaging

OHVIRA

Obstructed hemi-vagina, ipsilateral renal anomaly

Author contributions

Conceptualization: HTN and XHTM. Writing, review, and editing: HTN and XHTM.

Funding

This case report was not funded by any organization.

Availability of data and materials

All the images and medical information will be provided upon request.

Declarations

Ethics approval and consent to participate

Not applicable.

Consent for publication

Written informed consent was obtained from the patient’s legal guardian for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.ererer

Competing interests

There is no competing interests in this publication.